A 23- year- old man, presented to the emergency department with sudden dyspnea, without another symptom. On examination he had hypoxemia, and abolished breath sounds in the right lung. Skin evaluation revealed the presence of multiple small rounded papules of white coloration with follicular accentuation, located in the upper dorsal region of the thorax (Fig. 1A). Computed tomography (CT) of the chest showed right spontaneous pneumothorax and multiple thin-walled cysts in both lungs (Fig. 1B).